Browse

Search for: rheumatoid arthritis    methotrexate    autoimmune disease    biomarker    gene expression    GWAS    HLA genes    non-HLA genes   

ID PMID Title PublicationDate abstract
8283113 [Dynamic roles of the upper extremity during axillary crutch gait]. 1993 Nov To ambulate successfully with an axillary crutch, the upper extremities play an important part in supporting the body's entire weight. To evaluate the roles of the upper extremity during axillary crutch gait 15 normal adult subjects, 23 patients with an affected single leg, and 3 patients with rheumatoid arthritis (RA), were investigated. The gait patterns using crutches were: the one-leg swing-through crutch gait and three-point crutch gait for both normal subjects and patients with an affected single leg, and the three-point crutch gait for RA patients. The real-time motion analysis system "Quick-MAG", force plates, and a VTR system were used for data acquisition. The forces at the arm-pit and the hands during the swing-through crutch gait decreased when the cadence increased from 60 to 100 steps per minute. However, the floor reaction curve suggested distortion of the rubber at the crutch tip when the cadence reached 100. In the plane of progression during the crutch stance phase, the elbow flexion angle was about 14 degrees, and the wrist radial flexion angle about 23 degrees in the middle of the crutch stance phase. This position might be the best for efficient force exertion from the upper extremity. The unloading ratio during the three-point crutch gait was 35 percent in the normal subjects, 49 percent in patients with an affected single leg, and 14 percent in the RA patients. The results of the unloading ratio, indicated that elbow and wrist joint impairment prevented the RA patients from sustaining enough posture to support body weight during the three-point crutch gait.
8230023 Intraarticular injection of fibronectin fragments causes severe depletion of cartilage pro 1993 Aug We have reported that fibronectin fragments (Fn-f) cause cartilage damage in vitro by causing enhanced release of proteases. In order to determine whether the Fn-f can damage cartilage in vivo, we have injected native fibronectin (Fn) and Fn-f into adolescent rabbit knee joints. After 7 days, tissue was analyzed by histochemical and biochemical techniques and remaining proteoglycans quantified. Injection of 0.6 or 3 microM Fn-f caused up to a 70% loss in total cartilage proteoglycan while native Fn, rabbit serum albumin or an Arg-Gly-Asp-Ser synthetic peptide, derived from the cell-binding domain of Fn, did not cause damage. Our results suggest that this Fn-f/damage model may be useful for generating cartilage damage in vivo for other studies. Since Fn-f have been detected in synovial fluids from joints of patients with rheumatoid arthritis and osteoarthritis, our results are consistent with the notion that Fn-F mediated damage may occur in vivo.
8502628 [Synchronization of plasma exchange and cyclophosphamide in severe systemic diseases. A co 1993 Feb 27 Ten patients with severe systemic diseases, including systemic lupus erythematosus (n = 2), polymyositis (n = 2), essential mixed cryoglobulinaemia (n = 2), rheumatoid arthritis vasculitis (n = 3) and Wegener's granulomatosis (n = 1), were treated with 3 consecutive plasma exchanges synchronized with pulse cyclophosphamide. This therapeutic regimen was applied every 4 weeks initially and thereafter every 6 weeks in case of positive response after the first 3 cycles; it was combined in all patients with corticosteroid therapy. The treatment was administered for severe flare-up of the disease in 7 patients and for failure of previous treatments, including corticosteroids, cyclophosphamide and plasmapheresis, in 3 patients. Three kinds of response were observed: lasting complete remission without relapse after synchronization had ceased in 4 patients, partial clinical remission with post-synchronization relapse in 5 patients, and primary failure without any clinical response to treatment in only 1 patient. These results suggest that repeated plasma exchanges synchronized with cyclophosphamide are effective against progressive autoimmune diseases and in cases where conventionally administered immunosuppressive treatments had failed. However, this type of treatment cannot prevent long-term relapses, and only a prospective study can evaluate its success in terms of survival.
8317328 Production of TNF alpha by LPS-stimulated murine, rat and human blood and its pharmacologi 1993 Tumour necrosis factor alpha (TNF alpha) has been reported to play a key role in the pathogenesis of sepsis and chronic inflammatory diseases, including rheumatoid arthritis and atherosclerosis, suggesting that agents which inhibit TNF alpha production may have therapeutic utility for the treatment of such conditions. Production of TNF alpha by LPS (lipopolysaccharide)-stimulated murine, rat and human heparinized blood was investigated. LPS (1-100 micrograms/ml) caused a similar concentration- and time-dependent stimulation of TNF alpha production by rat and human blood, achieving levels of 750-5000 U/ml (L929 bioassay) at 6 h. In contrast, TNF alpha production by LPS-stimulated murine blood was poor and variable (0-150 U/ml). Dexamethasone and pentoxifylline caused a concentration-dependent inhibition of TNF alpha production by LPS-stimulated human and rat blood with IC50s of 0.26 +/- 0.05 and 73.0 +/- 26.4 microM for human and 5.7 +/- 1.8 nM and 20.6 +/- 8.0 microM for rat blood, respectively. Therefore, LPS-stimulated rat and human, but not murine, blood are suitable systems for the detection and evaluation of inhibitors of TNF alpha production.
7680981 Nabumetone. A reappraisal of its pharmacology and therapeutic use in rheumatic diseases. 1993 Jan Nabumetone is a nonsteroidal anti-inflammatory drug (NSAID) used to treat rheumatic and inflammatory conditions. It is absorbed as a nonacidic prodrug and is rapidly converted in the liver to an active metabolite which is responsible for its anti-inflammatory and analgesic effects. Published data from earlier comparative studies indicate that nabumetone, administered in a single dose of 1 to 2g daily, is as effective as aspirin, diclofenac, ibuprofen, indomethacin, naproxen and sulindac for the symptomatic treatment of rheumatoid arthritis, osteoarthritis, various nonarticular rheumatic conditions and acute soft tissue injury. Adverse events with nabumetone occur less frequently than with aspirin, and the incidence of gastrointestinal adverse events with nabumetone compares favourably with that of other NSAIDs. Rates of gastrointestinal ulceration and bleeding with nabumetone are low, apparently less than 1% annually. More recently, data from large-scale clinical trials and postmarketing surveillance studies have further confirmed the efficacy and tolerability of nabumetone. Thus, the drug should now be considered a well established member of this group of agents for the treatment of painful rheumatic and inflammatory conditions.
1432300 Mushroom worker's lung. Detection of antibodies against Shii-take (Lentinus edodes) spore 1992 Nov Indoor cultivation of the edible mushroom Shii-take (Lentinus edodes) regularly leads to symptoms of mushroom worker's lung (MWL) in workers. An immunologic test is described allowing detection of IgG type antibodies against Shii-take spore antigens. It was found that MWL patients employed in Shii-take picking (n = 5) have significantly increased antibody titres against Shii-take spore antigens. Different control groups, viz, MWL patients employed in the cultivation of the white button mushroom Agaricus bisporus (n = 14) and of the oyster mushroom Pleurotus spp (n = 3), patients with Bechterew's syndrome (n = 7), sarcoidosis (n = 7), rheumatoid arthritis (n = 9), and healthy controls were found in the same range of low titres. The use of protective masks during picking reduced complaints of the workers (n = 14). However, their antibody titres increased with duration of employment despite the protection.
1306121 Genotyping human polymorphic arylamine N-acetyltransferase: identification of new slow all 1992 Oct Arylamine N-acetyltransferase catalyses the N-acetylation of primary arylamine and hydrazine drugs and chemicals. N-acetylation is subject to a polymorphism and humans can be categorized as either fast or slow acetylators according to their ability to N-acetylate polymorphic substrates in vivo. Previously, slow acetylation has been linked to four distinct polymorphic N-acetyltransferase (pnat) alleles each of which contains one or more point mutations within the coding region of the pnat gene. One new rare slow variant of pnat has been identified by cloning and sequencing the pnat DNA from an individual whose NAT phenotype was determined by in vivo acetylation of the polymorphic substrate sulphamethazine. This allele, designated S1c, differs from the wild type fast allele at nucleotide positions 341 and 803. A second new rare slow allotypic variant, designated S3, has been identified by resistance of the pnat specific DNA to digestion with the restriction enzymes Fok I and Bam HI. A method of genotyping individuals for the arylamine N-acetyltransferase (NAT) polymorphism is presented which correctly predicts the phenotype of greater than 95% (21 of 22) of individuals as measured by the extent of acetylation of sulphamethazine in urine. This refined genotyping method was applied to a clinical population of 48 Caucasians with classical or definite rheumatoid arthritis each receiving daily between 150 and 500 mg of the anti-rheumatic drug, D-penicillamine. There is no difference in the N-acetyltransferase phenotype of the individuals who developed proteinuria and the control group with no adverse effects.
1403320 [A report of two cases of cervical necrotizing soft-tissue infection]. 1992 Sep Two recent cases of cervical necrotizing soft-tissue infection are herein presented. Case 1. A 52-year-old man with uncontrolled diabetes was hospitalized because of an erythematous swelling of the left side of his neck and high grade fever. Fetid yellowish pus exuded from the left parotid area. The swelling extended from the left temporal area to the left supraclavicular fossa, with necrosis of the parotid gland, sternocleidomastoid, masseter and a portion of the strap muscles. Wound cultures revealed Staphylococcus aureus and alpha-hemolytic streptococcus. No anaerobic bacteria were detected. Treatment consisted of intravenous administration of antibiotics, control of diabetes with insulin, and debridement of the necrotic tissue, which left an epidermal defect in the initially swollen area. Transfer of a forearm free flap was done after the growth of healthy granulation tissue over the affected area. Case 2. A 55-year-old woman with rheumatoid arthritis was transferred to our hospital after tracheotomy performed in another hospital because of dyspnea due to severe crepitant swelling of her cheeks and submandibular areas bilaterally, and her left temporal area. A copious amount of fetid pus exuded from the incisions made in the left temporal area, left cheek, and right submandibular area. There were bilateral diffuse rales. Culturing the pus revealed alpha-hemolytic streptococci, while MRSA and Pseudomonas aeruginosa were detected from cultures of sputum. No anaerobic bacteria were found. After intravenous administration of antibiotics, infected wounds and pneumonia were ameliorated, and necrotic subcutaneous tissue and fascia were debrided. The patient was discharged with a residual depression in her left cheek and a scar on her left temporal area.
1645029 Cytokines and local factors which affect osteoclast function. 1992 Jul Bone remodeling is a local phenomenon which occurs in discrete packets throughout the skeleton. The cellular events which comprise the remodeling sequence are controlled by cytokines which are generated in the microenvironment of the bone resorbing pockets. These cytokines are derived from marrow mononuclear cells or from bone cells themselves, or they are incorporated into the bone matrix and released in biologically active form as bone resorbs. Evidence is accumulating that some of these cytokines play an important role not just in physiological bone remodeling, but also in common diseases of bone remodeling such as osteoporosis, osteopetrosis, Paget's disease, and malignant diseases which involve bone and chronic inflammatory diseases such as rheumatoid arthritis and periodontal disease. Normal bone remodeling is clearly under local control. It occurs in discrete packets throughout the skeleton, each of which is geographically distinct. Local packets of bone remodeling are also asynchronous with respect to each other. The cellular events which comprise the remodeling sequence are thus regulated primarily by factors which are enriched in that microenvironment. The remodeling sequence, which is continuous, is the same on cancellous bone surfaces as it is within the Haversian systems of cortical bone. Since it is now known that powerful osteoclastotropic factors are produced in the microenvironment of these bone remodelling packets, these local factors or cytokines are the most likely major regulators of osteoclast function.
1325849 Host modulation with tetracyclines and their chemically modified analogues. 1992 Mar Recent studies have suggested the use of drugs to modulate host response as a new approach in periodontal therapy. In this regard, the tetracycline antibiotics have been found to inhibit host-derived collagenases and other matrix metalloproteinases by a mechanism independent of the antimicrobial activity of these drugs; this effect may suppress connective tissue breakdown during periodontal disease and during a variety of medical disorders including (but not limited to) noninfected corneal ulcers, serious (sometimes life-threatening) skin-blistering diseases, rheumatoid arthritis and osteoarthritis, systemically--as well as locally--induced bone loss, and perhaps even tumor-induced angiogenesis. Two therapeutic strategies based on the host-modulating properties of tetracyclines are currently being developed: 1) the use of low-dose doxycycline (the most potent anticollagenase of commercially available tetracyclines) formulations, which do not appear to result in tetracycline side effects such as the emergence of antibiotic-resistant microorganisms; and 2) the production of a family of chemically modified tetracyclines that have lost their antimicrobial activity, but have retained their anticollagenase activity. A description of several of these compounds and a discussion of their efficacy in inhibiting collagenases in vitro and reducing tissue destruction in several animal models of periodontal and medical diseases is presented.
1446975 Reactive oxygen species as regulators of human neutrophil and fibroblast interstitial coll 1992 The effects of various reactive oxygen species on latent human neutrophil and fibroblast-type interstitial collagenases were studied. Latent human neutrophil collagenases (proMMP-8) was efficiently activated by hypochlorous acid and hydrogen peroxide and less efficiently by the serine proteinases trypsin and chymotrypsin. Human plasmin and plasma kallikrein did not activate latent human neutrophil collagenase. The activation of latent human neutrophil collagenase by hypochlorous acid and hydrogen peroxide corresponded to the activation obtained with the other known non-proteolytic activators phenylmercuric chloride and gold thioglucose. The activation by hydrogen peroxide was inhibited by mannitol and desferoxamine, suggesting a localized Fenton-type reaction to be responsible for the generation of hydroxyl radical and/or hydroxyl radical-like reactive oxygen pathway of neutrophil procollagenase does not involve plasmin and plasma kallikrein, which are efficient proteolytic activators of latent fibroblast-type procollagenase (proMMP-1). Fibroblast procollagenase was also slightly activated by hypochlorous acid and gold thioglucose. Thus neutrophil procollagenase seems to prefer non-proteolytic means of activation and reactive oxygen species can be regarded as potent activators in vivo. Synovial-fluid neutrophils from rheumatoid arthritis patients were found to release collagenase in 30% active form when compared to same patients' peripheral blood neutrophils, which released collagenase in completely latent form. This may indicate that the triggering of neutrophil at the site of inflammation in vivo involves initial oxidative activation of collagenase upon the degranulation process.
8970041 Comparative study of 4 diagnosis criteria sets for mixed connective tissue disease in pati 1996 Dec OBJECTIVE: To evaluate the performances of 4 sets of criteria proposed to define mixed connective tissue disease (MCTD): the criteria of Sharp, Alarcón-Segovia, Kasukawa, and Kahn. As anti-U1-RNP antibodies appear indispensable to establish the diagnosis of MCTD, we wished to reevaluate these sets of criteria in patients who all had anti-U1-RNP antibodies. METHODS: We analyzed clinical and biological data to find which diagnostic criteria were met by 45 patients with anti-U1-RNP antibodies. We tested criteria for rheumatoid arthritis, systemic lupus erythematosus, systemic sclerosis, polymyositis, Sjögren's syndrome, and 4 sets of criteria for MCTD. RESULTS: The criteria that best identified patients with MCTD were those proposed by Alarcón-Segovia, with 62.5% sensitivity and 86.2% specificity, comparable to Kahn's criteria. The overlap with other connective tissue diseases was found to be 16%. These results could be improved by using the term "myalgia" instead of "myositis" in the definition. This increased sensitivity to 81.3%, with no decrease in specificity. CONCLUSION: Alarcón-Segovia's and Kahn's criteria are the best classification criteria to define MCTD.
8844971 Molecular mechanism of transcriptional activation of human gelatinase B by proximal promot 1996 Sep 10 Gelatinase B is a regulated matrix metalloproteinase with important role in the remodeling of extracellular matrix and many pathological conditions such as tumor invasion and rheumatoid arthritis, physiological processes including embryonic growth and development, migration of blood leukocytes into tissues and tissue remodeling. Elevated levels of certain MMPs are believed to be associated with various pathological states. We cloned the 5'-flanking 600 bp sequence of human gelatinase B gene by PCR, which controls the expression of the gene by ligating it to the chloramphenicol acetyltransferase gene. Four kinds of cell lines were used to transiently transfect. Deletion analysis revealed that 100 bp (-600 to -500 bp) contributed positively to induction by tumour necrosis factor. The 100 bp contains NF-kappa B site, Ap-1 site, PEA3 and Sp-1 site. The expression of the human gelatinase B gene varied in different cells in the presence of TNF.NF-kappa B factor may play an important role in regulating the gene expression. Comparison of the finding with those for the promoter of gelatinase A, collagenase and stromelysin shows that the determinant for the inducibility of the gelatinase B gene is more complex.
8671616 Antigen processing and presentation by a mouse macrophage-like cell line expressing human 1996 Mar Macrophages differ from other antigen-presenting cell types in their potential to take up, process and present particulate antigens as well as proteins and peptides. To study their influence on T cell activation we transfected the mouse macrophage-like cell line P388D1 with human genes coding for the alpha and beta chains of class II molecules (DRA*0101 and DRB1*0401 or DRB1*0404) or with a series of localized DR beta mutants. The transfected cells (TP cells) all expressed DR4 molecules on their surface. Since they stimulated some, but not all, human DR4-reactive T cell clones, some of the resident peptides are evidently similar in mouse and man. Proteins and polypeptides such as pepsin or the human acetylcholine receptor (AChR) alpha 37-181 were also processed correctly by these transfectants and then presented efficiently to other T cell clones. Nearly all the mutants tested could present to the pepsin-specific clone, establishing functional expression of the transfected DR molecules. For the more exacting AChR alpha 144-156-specific T cell clone PM-A1, we confirmed that the single Gly86--> Val mutation in the DR beta chain abolished presentation by two DR4 subtypes. If, however, this same replacement was made in a third variant that has Lys71 (rather than Arg71), the effects were less drastic. This approach could also be used to analyse the contributions of individual substitutions that confer susceptibility to rheumatoid arthritis.
8935055 Implications of temporomandibular disorders for facial growth and orthodontic treatment. 1995 Dec The purpose of this article is to review the literature concerning the possible associations between temporomandibular disorders (TMD), orthodontic therapy, mandibular growth, and facial form. Consideration of the association between TMD and orthodontic treatment leads to one conclusion: there is no evidence that orthodontic treatment generally increases or decreases the chances of developing TMD later in life. Still, our understanding of TMD is not final, and the expanding diagnostic knowledge continues to call for new longitudinal studies focusing on the developmental basis of temporomandibular disorders. Little is known about the potential effect of TMD on facial development. However, information relating to the normal development, growth, and adaptation of the temporomandibular joint all tend to emphasize the significant ontogenetic plasticity of the growth-related secondary cartilage associated with the TMJ, within the bounds of normal function and histophysiology. With the exception of such diseases as juvenile rheumatoid arthritis and osteoarthritis, little is known about the influence of TM pathology or myofascial disorders on facial growth. Also, little is known about the possible influence of disc interferences on facial growth. However, the condyle is known to play a prominent role in normal mandibular growth and, consequently, facial development. Thus, categories of TMD that involve dysplasia of the condylar cartilage could be associated with aberrant facial growth and form.
7496138 Glycoforms of serum alpha 1-acid glycoprotein as markers of inflammation and cancer. 1995 Jun alpha 1-acid glycoprotein (AGP) is a serum acute phase glycoprotein which possesses five N-linked complex type heteroglycan side chains which may be present as bi-, tri- and tetraantennary structures. Depending upon the content of biantennary structure on AGP, up to four glycoforms of AGP are present in serum. These glycoforms can be easily estimated in body fluids by means of crossed affinity-immunoelectrophoresis (CAIE) with the lectin, Concanavalin A (Con A). Con A selectively binds biantennary structures; the more biantennary structures on AGP, the stronger the binding. In acute inflammation, a relative increase of AGP glycoforms with biantennary units is observed-a type I glycosylation change. In some chronic inflammatory states there is an relative decrease of AGP glycoforms with biantennary heteroglycans-a type II glycosylation change. Moreover, in certain other states such as pregnancy, estrogen administration or liver damage, type II glycosylation changes are also seen. A detailed analysis of the clinical applications of the assessment of AGP glycoforms in sera of patients with rheumatic diseases, AIDS and various types of cancers is presented. Accumulated data shows that AGP glycoforms may be very useful in the detection of intercurrent infections in the course of rheumatoid arthritis, systemic lupus erythematosus, or myeloblastic leukaemia, and in the detection of secondary infections in human immunodeficiency virus infected individuals. AGP glycoforms are also very useful in differentiation between various forms of trophoblastic disease and are helpful in monitoring the treatment of these patients. Finally, AGP glycoforms provide valuable information for differentiation between primary and secondary liver cancer.
7660343 Sclerosing mediastinitis: a report on 18 cases. 1995 Mar BACKGROUND: Sclerosing mediastinitis is a rare condition which causes dense fibrosis of the mediastinum. Few large studies have been reported to date. The clinical and pathological features of cases have been studied in a specialist referral centre in the UK. METHODS: The pathological files of the Royal Brompton Hospital were examined and 18 cases of sclerosing mediastinitis were identified between 1970 and 1993. The clinical notes were obtained and the pathological specimens analysed. RESULTS: There were 12 men and six women of age range 9-64 years. Twelve patients presented with shortness of breath, six had haemoptysis, three had hoarseness, four had pleuritic chest pain, three general weakness, two had dysphagia, and one was asymptomatic. Nine patients had a previous history of pulmonary tuberculosis. Two had auto-immune disease--one rheumatoid arthritis and the other systemic lupus erythematosus. There were three cases of previous malignancy--two undifferentiated carcinoma of the lung and the other Hodgkin's disease. Serological tests revealed only one positive reaction to Histoplasma. The erythrocyte sedimentation rate and serum immunoglobulins were raised in nine patients. Diagnosis was usually by thoracotomy with biopsy. All cases had fibrosis and chronic inflammation with no active granulomas. No infective organisms or positive cultures were obtained in any case. Ten of the 18 cases are alive up to 15 years after diagnosis, with only two deaths and six lost to follow up. CONCLUSIONS: Sclerosing mediastinitis is a slowly progressive condition associated with previous tuberculosis, mediastinal malignancy, and autoimmune disease. The outlook is excellent for those cases without underlying malignancy.
7724995 Influence of interleukin-1 beta, tumour necrosis factor alpha and prostaglandin E2 on chon 1995 Inflammatory mediators such as the cytokines interleukin-1 (IL-1) or (TNF alpha), and prostaglandins [predominantly prostaglandin E2 (PGE2)] are generally considered to be involved in the breakdown of cartilage matrix in chondrodestructive diseases, especially rheumatoid arthritis and osteoarthritis. Their mode of action is not yet completely understood. Blastemal cells or differentiated chondroblasts/chondrocytes of limb buds from mouse embryos (day 12) in organoid cultures provide an efficient system to investigate the mechanism of action of these substances. Using recombinant human IL-1 beta, TNF alpha and PGE2 alone or together (in pairs) in this culture system, we found that none of these substances alone could affect chondrogenesis. TNF alpha, however, when combined with IL-1 beta, proved to be the more potent cytokine causing a transformation of embryonal chondrogenic cells into fibroblast-like cells and thus inhibiting the expression of the cartilage cell phenotype. This might be due to inhibition of both the morphogenetic and cytodifferentiation phases of chondrogenesis. The well-known synergistic interaction between both cytokines seems to be phase limited and may not occur in the postchondrogenesis phase. In addition, our results showed that TNF alpha alone or combined with PGE2 caused a marked breakdown of the cartilage matrix. These in vitro findings might be useful to elucidate the complexity of interactions between different cytokines and PGE2 involved in cartilage destruction processes in vivo.
7806541 Structure of a novel phosphocholine-containing glycoglycerolipid from Mycoplasma fermentan 1994 Dec 30 Mycoplasma fermentans is thought to be a pathogen of rheumatoid arthritis or cofactor of AIDS. A novel phosphocholine-containing glycoglycerophospholipid named GGPL-I was isolated from a M. fermentans-infected human helper T-cell culture. It was revealed that GGPL-I is a lipid component of the M. fermentans and a major immunological determinant. The GGPL-I was purified by DEAE-Sephadex column chromatography and repeated Iatrobeads column chromatography. The purified glycophospholipid was subjected to structural characterization by thin-layer chromatography, Fourier-transform infrared spectrometry, liquid secondary ion mass spectrometry, and nuclear magnetic resonance spectroscopy. Its structure was determined to be as follows: 6'-O-phosphocholine-alpha-glucopyranosyl-(1'-3)-1,2-diacyl-sn-glycerol. This glycoglycerophospholipid is unique in containing phosphocholine, which is attached to C-6 of glucose. The stereospecific numbering (sn) of naturally occurring GGPL-I was determined through comparison with chemically synthesized compounds.
7967763 T-cell large granular lymphocytic leukemia and pure red cell aplasia in a patient with typ 1994 Nov Clonal proliferations of large granular lymphocytes (LGLs) of T-cell origin characterize T-cell LGL leukemia. This disorder has been described in association with rheumatoid arthritis and other autoimmune phenomena. The presence of endocrinologic abnormalities in patients with T-cell LGL leukemia has not been previously reported, nor has T-cell LGL leukemia been described in patients with endocrinologic abnormalities. Herein we describe a young woman with type I autoimmune polyendocrinopathy, in whom pure red cell aplasia developed in association with clonal proliferation of LGLs. Immunosuppressive therapy with cyclophosphamide resulted in remission of pure red cell aplasia, transient improvement in hypocalcemia, and disappearance of the LGL clone. Clonal proliferation of LGLs may be associated with autoimmune endocrinopathies. Clinicians who are responsible for the care of such patients should be aware of this possible association.